Article
A 30-amino acid truncation of the microsomal triglyceride transfer protein large subunit disrupts its interaction with protein disulfide-isomerase and causes abetalipoproteinemia.
The Journal of biological chemistry - 16 Jun 1995
Ricci B, Sharp D, O'Rourke E, Kienzle B, Blinderman L, Gordon D, Smith-Monroy C, Robinson G, Gregg R E, Rader D J
Abstract excerpt
The microsomal triglyceride transfer protein (MTP) is a heterodimer composed of the multifunctional enzyme, protein disulfide-isomerase, and a unique large, 97 kDa, subunit. It is found as a soluble protein within the lumen of the endoplasmic reticulum of liver and intestine and is required for t...
Topics
- Abetalipoproteinemia
- Base Sequence
- Carrier Proteins
- Cell Line
- Cholesterol Ester Transfer Proteins
- DNA Primers
- Glycoproteins
- Humans
- Isomerases
- Male
- Middle Aged
- Molecular Sequence Data
- Mutation
- Nucleopolyhedroviruses